The most common form of macular telangiectasia is type 2. It affects middle-aged or older people and can lead to progressive vision loss. There is thickening of the basement membrane with a decrease of endothelial cells and pericytes, very similar to diabetic vascular change. Macular telangiectasia type 2 can be associated with intraretinal neovascularization, subretinal neovascular membrane, exudation, and hemorrhage.1
Foveal cyst formation, absent or minimal macular edema (despite prominent leakage on fluorescein angiography [FA]), intraretinal hyperreflective lesions, and foveal flattening are the most common optical coherence tomographic (OCT) findings in patients with macular telangiectasia type 2. These may represent progressive loss of retinal tissue, possibly due to Müller cell degeneration. Visual loss occurs from atrophy of the foveal retina rather than exudation or retinal edema. A subnormal foveal thickness in macular telangiectasia type 2 may be associated with better macular function as assessed by microperimetry. It is hypothesized that in macular telangiectasia type 2, both primary neurosensory thinning and low-grade macular edema can be involved. The coincidence may result in normal retinal thickness but decreased retinal function. The hyperreflective plaque with shadowing on OCT corresponds to a black foveal or juxtafoveolar pigment plaque.2
Laser treatment is not effective in macular telangiectasia type 2.3 There are case reports regarding the treatment of macular telangiectasia with photodynamic therapy, intravitreous drugs (especially triamcinolone acetonide, bevacizumab, and pegaptanib), and combination therapy.4,5
The purpose of this study is to describe a case series of 3 eyes treated with intravitreous ranibizumab for foveal detachment secondary to macular telangiectasia type 2.
The 2 patients were female and are described in the Table. All 3 eyes demonstrated leakage on FA and foveal detachment before the injection of ranibizumab. After discussing various treatment options and obtaining informed consent, a single dose of intravitreous ranibizumab (0.5 mg) was administered. Institutional review board approval was obtained before medical record review. One month and 6 months after the injection, all eyes demonstrated better visual acuity (VA) and reduced leakage on FA. This improvement in VA and FA results was associated with regression of foveal detachment on OCT. No treatment-related adverse effects were noted.
A 62-year-old woman had bilateral progressive decreased vision for 8 years and additional loss of vision and metamorphopsia in her right eye for 3 months. She has had hypertension and well-controlled diabetes since 2001. She did not have carotid artery disease, radiation retinopathy, diabetic retinopathy, or other retinal vascular disease. She was treated 8 months earlier with macular grid photocoagulation and ketorolac tromethamine, 0.4%, eyedrops in both eyes without improvement. Her best-corrected VA was 20/200 OD and 20/50 OS. The retinal examination showed bilateral macular telangiectasia with intraretinal pigment deposition and foveal detachment in the right eye. At the 4-week follow-up, VA improved by 2 Snellen lines (20/60). Fluorescein angiography showed a marked reduction of exudation, and OCT images showed marked reversal of the foveal detachment (Figure 1). At the 6-month follow-up, the VA and OCT results remained stable.
Right eye of a 62-year-old woman with macular telangiectasia type 2. A, Optical coherence tomographic image before treatment with intravitreous ranibizumab (0.5 mg). B, Optical coherence tomographic image at the 6-month follow-up showing marked reversal of the foveal detachment. C, Fluorescein angiogram before treatment. D, Fluorescein angiogram at the 6-month follow-up showing partial regression leakage.
A 46-year-old woman had macular telangiectasia type 2 for 6 years without prior treatment. Before the ranibizumab injection, her VA was 20/100 OD and 20/400 OS; OCT showed bilateral foveal detachment. Perifoveal leakage was detected on FA. One month after treatment of both eyes, VA was 20/60 OD and 20/80 OS, there was marked diminution of the central retinal thickness, and there was slight decreased leakage on FA (Figure 2 and Figure 3). At the 6-month follow-up, the VA and OCT results remained stable.
Right eye of a 46-year-old woman with macular telangiectasia type 2. A, Optical coherence tomographic image before treatment with intravitreous ranibizumab (0.5 mg). B, Optical coherence tomographic image at the 6-month follow-up showing marked reversal of the foveal detachment. C, Fluorescein angiogram before treatment. D, Fluorescein angiogram at the 6-month follow-up showing partial regression leakage.
Left eye of a 46-year-old woman with macular telangiectasia type 2. A, Optical coherence tomographic image before treatment with intravitreous ranibizumab (0.5 mg). B, Optical coherence tomographic image at the 6-month follow-up showing marked reversal of the foveal detachment. C, Fluorescein angiogram before treatment. D, Fluorescein angiogram at the 6-month follow-up showing partial regression leakage.
We describe 3 eyes (2 patients) with foveal detachment secondary to macular telangiectasia type 2 that underwent treatment with intravitreous ranibizumab (0.5 mg).
Grid laser photocoagulation therapy for macular edema in patients with macular telangiectasia appears to neither improve nor stabilize long-term VA. In addition, treatment is associated with retinal pigment epithelial changes, increased postoperative retinal vascular distortion, postoperative vascularized retinal scars, and postoperative retinal hemorrhages. These changes, however, do not appear to cause a further loss of vision.3
In nonproliferative macular telangiectasia, intravitreous bevacizumab decreases FA leakage but has no short-term effect on VA or OCT appearance. In proliferative macular telangiectasia, intravitreous bevacizumab arrests the leakage and growth of subretinal neovascularization with the possibility of VA improvement.5 Combined therapy with photodynamic therapy and intravitreous ranibizumab appears to be efficacious in the treatment of subretinal neovascular membrane associated with proliferative macular telangiectasia type 2.4
Ranibizumab was developed for the treatment of age-related macular degeneration by intravitreous administration. This antibody fragment inhibits all forms of biologically active vascular endothelial growth factor A. This fact has stimulated its use in other vascular diseases such as diabetic macular edema, retinal vein occlusion, telangiectasia, polypoidal choroidal vasculopathy, conjunctival neoplasia, and von Hippel–Lindau disease. However, we should always weigh the concern about the potential deleterious effects of repeated injections and chronic vascular endothelial growth factor suppression with the cost of therapy and the benefit to the patient.5
In conclusion, intravitreous ranibizumab as monotherapy appears to be efficacious in the treatment of foveal detachment secondary to macular telangiectasia type 2. Although these cases showed a favorable outcome, additional larger studies with longer follow-up are required.
Correspondence: Dr Lira, Rua Irmã Maria David, 200 ap 1302, Recife, Pernambuco, Brazil 52061-070 (firstname.lastname@example.org).
Author Contributions: All of the authors had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Financial Disclosure: None reported.
Lira RPC, Silva VB, Cavalcanti TM, de Souza ACD, Pinto APDC. Intravitreous Ranibizumab as Treatment for Macular Telangiectasia Type 2. Arch Ophthalmol. 2010;128(8):1075-1078. doi:10.1001/archophthalmol.2010.155